Aorto–Right Ventricular Tunnel Causing Functional Tricuspid Atresia

Burkhardt, Barbara; Arnold, Raoul; Schlensak, Christian; Bökenkamp, Regina; Stiller, Brigitte

doi:10.1161/circulationaha.111.062570

Cited by 6 publications

(14 citation statements)

References 15 publications

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“…Nevertheless, estimating the degree of tricuspid stenosis is difficult unless pulmonary regurgitation is eliminated. In a case similar to the present one, Barbara et al reported that an aorto-right ventricular tunnel caused functional tricuspid atresia 4 . In addition, the degree of tricuspid stenosis is closely related to single-ventricle or biventricular physiology.…”

Section: Discussionsupporting

confidence: 78%

“…In a case similar to the present one, Barbara et al reported that an aorto-right ventricular tunnel caused functional tricuspid atresia. 4 In addition, the degree of tricuspid stenosis is closely related to single-ventricle or biventricular physiology. In the present case, because main pulmonary artery ligation was performed and pulmonary regurgitation was absent, the tricuspid valve diastolic opening could be estimated.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Functional near-tricuspid atresia in a patient with absent pulmonary valve and an intact ventricular septum

2016

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Absent pulmonary valve with an intact ventricular septum is a rare malformation. We report a case of absent pulmonary valve and intact ventricular septum with functional near-tricuspid atresia caused by pulmonary regurgitation. Initial palliation with main pulmonary artery ligation and bilateral pulmonary artery banding was performed at 1 day of age. More antegrade flow across the tricuspid valve was recognised postoperatively, resulting in a successful right ventricular outflow tract reconstruction by a hand-sewn bileaflet polytetrafluoroethylene valve and modified Blalock-Taussig shunt at 11 days of age.

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Section: Discussionsupporting

confidence: 78%

Section: Discussionmentioning

confidence: 99%

Functional near-tricuspid atresia in a patient with absent pulmonary valve and an intact ventricular septum

2016

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“…5,9,14,15,18,39 –52,95 The specific anomalies reported include atresia of arterial orifice and origin of the artery involved from the proximal or distal end of the tunnel. 5,9,14,15,18,39 –52,95 More than two orifices, a single or stenotic orifice, and an intramural course of the coronary artery in the posterior wall of the tunnel have all been observed. 9,10,18,95 Other reported associated anomalies include aneurysms of the interventricular septum, ventricular septal defect, so-called “left ventricular noncompaction,” and transposition in a solitary case.…”

Section: Resultsmentioning

confidence: 99%

A Review of the Surgical Management of Aorto-ventricular Tunnels

Chowdhury

Anderson

George

et al. 2021

World J Pediatr Congenit Heart Surg

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We present a synthesis of 95 published investigations of the exceedingly rare tunnels that can exist between the aortic root and the left or right ventricles. From the 220 suitable cases included in these investigations, we reviewed the clinical presentations, modalities used for diagnosis, surgical approaches, and outcomes. Diagnostic information was provided by clinical presentation, radiographic findings, saline contrast echocardiography, computed tomographic angiocardiography, magnetic resonance imaging, cardiac catheterization, and angiocardiography. These techniques elucidated the coronary arterial origins and associated defects and defined the disease before surgery. Patients occasionally present with an asymptomatic cardiac murmur and cardiomegaly, but most suffer cardiac failure in the first year of life when the tunnel enters the left ventricle. Antenatal diagnosis by fetal echocardiography is reliable after 18 weeks of gestation. Associated defects, involving the proximal coronary arteries or the aortic or pulmonary valves, are present in nearly half the cases. Prompt diagnosis and surgical repair are important for a favorable outcome. Overall, operative mortality has been cited to be between 3% and 8.3%. Associated congenital coronary arterial anomalies, residual severe aortic stenosis, poor left ventricular function, and rupture of an infected suture line have been the reported causes of death. Despite early surgical intervention, an incidence of 16% to 60% postoperative residual aortic regurgitation of varying severity has been reported. The requirement of further repair or replacement of the aortic valve ranges from 0% to 50%. We submit that an increased appreciation of these details relative to the tunnels will contribute to improved surgical management.

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“…According to the literature most common tunnel orifice originating from the right coronary sinus (about 80% of cases) (2)(3)(4)(5)(6)(7)(8). In this case, it is necessary to differentiate with fistulas.…”

Section: Discussionmentioning

confidence: 99%

“…Aorto-right ventricular tunnel is an extremely rare anomalous communication between aorta and the right ventricular cavity (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18). This defect was first reported by K. Hart (1905) and J.…”

Section: Introductionmentioning

confidence: 99%